Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0149514 (bronchitis)
 6,902 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 39-year-old man with a history of repeated attacks of acute bronchitis and pneumonia since childhood was admitted complaining of cough, sputum, and a high fever. A diagnosis of Kartagener's syndrome was made because of the presence of mirror-image dextrocardia with complete situs inversus, bronchiectasis in right B6 and B10, and chronic sinusitis. Examination of the ciliary ultrastructure of the bronchial epithelium showed defective inner dynein arms and impaired nasal clearance was suggested by the saccharin particle method. Contrast venography of great veins revealed a McCotter type II bilateral superior vena cava and an absent inferior vena cava with (hemi-) azygos continuation. A search to find a similar case in the literature failed. Abnormal ciliary movement from the embryonic period was implicated as a causative factor in Kartagener's triad and the morphological abnormalities of the great veins.
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PMID:[A case of Kartagener's syndrome with bilateral superior vena cava and absent inferior vena cava]. 186 4

Primary ciliary dyskinesia represents a group of heritable disorders of cilia and sperm affecting between 1 in 15,000 and 1 in 30,000 persons. Those affected lack measurable mucociliary clearance and suffer the constant misery of rhinorrhea and chronic productive cough. Because mucociliary clearance constitutes one of the respiratory system's major lines of defense, these patients are vulnerable to chronic sinusitis, bronchitis, pneumonia, and otitis media. Left untreated, these problems may progress to bronchiectasis, found frequently in adult patients, or pulmonary hypertension with eventual cor pulmonale. Screening for this disorder includes some simple and inexpensive methods as well as more exotic techniques requiring special camera equipment and an electron microscope to make a definitive diagnosis. Physiotherapy techniques can be taught to patients with primary ciliary dyskinesia and go a long way toward making up for the lack of mucociliary clearance. Vigorous bronchopulmonary toilet and palliative measures may enable these patients to enjoy relatively normal lives.
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PMID:Primary ciliary dyskinesia. 194 76

A case of immotile cilia syndrome (ICS) is presented. A 34-year-old male, who had suffered from recurrent bronchitis, sinusitis and otitis media since early childhood, was admitted to Keio University Hospital complaining of productive cough and infertility. A saccharin test showed prolonged nasal clearance time, and semen analysis revealed immotile sperm. By electron microscopic observation of cilia of the nasal mucous epithelium and the sperm, inner and outer dynein arm defect, abnormal microtubular arrangement and compound cilia were detected and he was diagnosed as ICS. Thirty eight Japanese cases of ICS collected from the literature were analyzed concerning clinical manifestations, airway clearance, family history and ciliary ultrastructural abnormalities (Table 2, 3). Recurrent bronchitis, male infertility, chronic sinusitis, otitis and decrease in airway clearance were very common in these patients. Female infertility was more common than anticipated. The prevalence rate of situs inversus was more than 50%, probably due to more attention having been paid to Kartagener's syndrome in Japan. Recent studies show that the cilia of this syndrome is not always immotile but characterized by hypomotility or asynchrony, and have attempted to determine the relationship between each ciliary ultrastructural abnormality and motility pattern. It is necessary that more patients including incomplete and mild cases should be investigated.
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PMID:[A case of immotile cilia syndrome and a review of Japanese cases]. 267 60

It is claimed that 25% of the total population show symptoms of a muco-ciliary chronic sinusitis, presenting as a posterior catharrh that can be confirmed by computerized tomography and by prolonged sinuso-nasal saccharine clearance. This manifestation of the mucus ciliary transport syndrome may be associated with other disorders (rhinitis, otitis, bronchitis, irritable colon, sterility and dextrocardia). The role of aspirin and intolerance to other drugs or chemicals especially anti-inflammatory drugs, has been implicated. Acid air pollution may play a part, as the respiratory mucus does not tolerate an acid pH. Alkaline aerosols and theophyllin which activates cilia mobility, usually increase mucus fluidity and facilitate its clearance from the sinuses.
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PMID:From Fernand Widal rhinitis syndrome and chronic sinusitis to total muco-ciliary disease. 322 58

Patients with common variable hypogammaglobulinemia (CVH) are susceptible to recurrent respiratory infections. We studied the prevalence and severity of respiratory dysfunction in 32 patients with CVH by measuring routine pulmonary function tests (PFT) and interpreting chest radiographs using a quantitative score system. Pulmonary symptoms were determined by patient interview and standard questionnaire. The mean age of 29 living patients was 28 +/- 2.2 yr; there were 22 males and 10 females. The mean age at diagnosis was 17.3 +/- 2.4 yr. Sinopulmonary symptoms were present in all 32 patients and included chronic sinusitis, otitis, bronchitis, recurrent pneumonia, and bronchiectasis. Complete PFT in 25 patients revealed restrictive, obstructive, and normal patterns in 10, 6, and 9 patients, respectively. Nine patients had dyspnea on exertion. Recent chest radiographs in 32 patients showed evidence of recurrent pulmonary infection, including air trapping, linear markings, and pleural abnormalities. Panlobular emphysema, documented in one case at autopsy, was suggested in 5 other patients by the presence of lower lobe bullous lesions and marked lower lobe hyperlucency on chest radiographs. The forced vital capacity (FVC) and forced expiratory volume in 1 second (FEV1) had remained stable in 19 adults (18 receiving adequate immunoglobulin replacement therapy) for 6.9 +/- 0.7 yr. The expected increase of the FEV1 and FVC with growth was normal in 6 of 8 children (all 8 receiving adequate therapy). Chest radiograph scores deteriorated only slightly from 20.3 +/- 0.6 to 18.4 +/- 0.8 (25 = normal) during the 6.9 +/- 0.7 yr in 30 patients (p less than 0.01).(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Respiratory dysfunction in patients with common variable hypogammaglobulinemia. 349 Feb

Cough is a common symptom in the smoking and non-smoking patient seeking medical attention from the office-based physician. Often, a comprehensive history and physical examination suggest the correct diagnosis, and specific therapy can be directed to the underlying disease. A chest roentgenogram is an essential part of the workup; it may suggest tuberculosis, chronic fungal infection, bronchiectasis, or lung abscess. In addition, bronchogenic carcinoma, which is increasing in frequency in the population, has several common manifestations that can be recognized on the chest roentgenogram. Pulmonary function studies are often helpful in the workup of the patient with chronic cough. A pattern of obstructive lung disease is seen with asthma, chronic bronchitis, and bronchiectasis. Diseases that cause lung fibrosis, such as idiopathic pulmonary fibrosis, sarcoidosis, and pneumoconiosis, give a restrictive ventilatory defect. Bronchoprovocation testing can be helpful when baseline pulmonary function tests are normal and the diagnosis of postviral bronchitis or cough-variant asthma is suggested. If the bronchial inhalation challenge is negative, these diagnoses can be excluded. Chronic rhinosinusitis with associated postnasal drip is one of the most common causes of chronic cough and is often difficult to confirm because the physical examination and roentgenogram of the paranasal sinuses may be normal. In a great majority of patients with chronic cough, a diagnosis can be established by simple, clinical and laboratory procedures used in the outpatient setting.
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PMID:Chronic cough. Diagnosis and treatment. 384 18

The immotile cilia syndrome (ICS) comprises a range of congenital defects of the ciliary apparatus most probably transmitted by autosomal recessive inheritance. Because cilia occur mainly in the respiratory and genital tract, the clinical symptoms of ICS are most commonly chronic sinusitis, bronchitis, bronchiectasis and male sterility. The syndrome can be associated with a situs inversus and is then called Kartagener's syndrome. We studied the ciliary ultrastructure in airway biopsies of 5 patients suffering from chronic upper and lower respiratory tract infections. With the single exception of one female patient with confirmed ICS diagnosis (Kartagener's syndrome) the etiology of the recurrent infections was unknown. The following ciliary defects were observed: missing dynein arms, radial spoke defects, missing nexin links, microtubular transpositions, compound cilia, supernumerary, absent, or incomplete microtubules, lack of ciliary orientation and various abnormal patterns of microtubular arrangement. In no instance did a patient show only a single anomaly; defects were always combined. Missing dynein arms, radial spoke defects and microtubular transpositions have frequently been described as lesions specific for ICS. Whenever these lesions were found simultaneously in both the respiratory and genital tracts, their genetic origin cannot be doubted. In our confirmed ICS patient the outer dynein arms were not missing but were reduced in number and length in a large number of cilia. The biopsy was, however, obtained from the heavily infected maxillary sinus and it is known that inflammation can lead to a loss of dynein arms. In the light of our investigations and of a review of the published cases of ciliary anomalies, it is concluded that none of the above defects in itself is specific for ICS. They may all occur as secondary lesions or sporadically as varieties in otherwise healthy subjects. It therefore appears questionable whether ICS can be diagnosed from the ciliary ultrastructure of a single airway biopsy. Assessment of ICS cannot be based simply on the ultrastructural demonstration of a particular ciliary defect, but necessitates additional considerations particularly regarding the origin of the biopsy, the sampling procedures and quantitation of defects. It appears necessary to investigate samples from different parts of the airways and quantitatively analyze the prominent lesions.
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PMID:[How useful is the ultrastructural study of the cilia of the respiratory tract in the diagnosis of an immotile cilia syndrome?]. 637 86

The function and ultrastructure of nasal cilia in 2 siblings with unexplained chronic sinusitis and bronchitis were investigated. Both patients lacked nasal mucociliary clearance (greater than 30 min). On electron microscopy, the ultrastructure of nasal cilia showed an extra doublet or supernumerary microtubules with disorganized microtubular network. Other axonemal components were normally present. It is conceivable that an "extra" component of the axoneme may be added to those "defects" already recognized as a cause of the "immotile-cilia syndrome".
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PMID:Supernumerary microtubules in the cilia of two siblings causing "immotile cilia syndrome". 665 89

In the immotile cilia syndrome, transmission electron microscopy of the cilia shows abnormalities in the arrangement of the central pairs of tubules and in the dynein arms of the peripheral tubules, or in the radial spokes, We studied four nonrelated children, 9/12, 5, 6, and 6 years old, with situs inversus and a history of chronic sinusitis and bronchitis (Kartagener's syndrome) and four children in the same age group and with the same history, but without situs inversus. Under the phase contrast microscope no motile cilia were seen in the four patients with Kartagener's syndrome and in two of the four other children. Transmission electron microscopy showed aberrations in the cilia (absence of dyneim arms, random orientation of central tubules) in the patients with Kartagener's syndrome. Scanning electron microscopy revealed differences in morphology and arrangement of cilia between patients and controls. In the patients much more mucus was present on the mucosal surface. Furthermore, the cilia were in a state of disorder, with a multidirectional orientation instead of the parallel orientation seen in controls.
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PMID:The immotile cilia syndrome: phase contrast light microscopy, scanning and transmission electron microscopy. 696 48

Mucociliary function was examined to investigate the association of chronic inflammation in the upper and lower airways in four groups of subjects. They were composed of: 1) non-inflammatory subjects (control group), 2) patients with chronic sinusitis (S group), 3) patients with chronic bronchitis (B group), and 4) patients with chronic sinusitis and bronchitis (SB group). Mucociliary clearance (MCC), ciliary beat frequency (CBF) in the nose and left main bronchus and dynamic viscoelasticity (DVE) of these airway fluids were examined. Significant prolongation of nasal MCC in the S and SB groups were observed compared with the control group. CBF in the nose of the S, B, and SB groups were not different from that of the control group. DVE of nasal mucus in the S, B, and SB groups were higher than that in the control group. Significantly diminished bronchial MCC in the B and SB groups were observed compared with the control group. CBF of the bronchus was within normal range in all examined subjects. DVE of tracheobronchial mucus in the S, B, and SB groups were higher than that in the control group. It was suggested that high viscoelasticity of the tracheobronchial mucus in patients with chronic sinusitis and of the nasal mucus in patients with chronic bronchitis may be one of the causes of sinobronchitis.
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PMID:Mucociliary function of chronic inflammation in upper and lower airways. 777 23


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